Skip to main content
SpringerLink
Log in

Vitreoretinal Lymphoma

  • Living reference work entry
  • First Online:
The Uveitis Atlas

  • 196 Accesses

Abstract

Primary vitreoretinal lymphoma (PVRL) is a variant of primary central nervous system lymphoma (PCNSL), mostly a high-grade, extra-nodal, non-Hodgkin’s lymphoma. Diffuse large B-cell lymphoma (DLBCL) represents the predominant subtype, while rare cases of T-cell and natural killer (NK) cell origin can occur. The hallmark clinical features include vitreous cells and subretinal pigment epithelial (RPE) infiltrates of lymphoma. PVRL frequently masquerades as uveitis, therefore establishing the diagnosis can be challenging. Vitreous or subretinal biopsy remains the gold standard for diagnosis. The majority of patients with PVRL ultimately develop PCNSL which is an aggressive malignancy associated with poor overall survival.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

Suggested Reading

  • Ahluwalia MS, Peereboom DM. Primary central nervous system lymphoma. Curr Treat Options Neurol. 2010;12:347–59.

    Article  PubMed  Google Scholar 

  • Chan CC, Sen HN. Current concepts in diagnosing and managing primary vitreoretinal (intraocular) lymphoma. Discov Med. 2013;15:93–100.

    PubMed  PubMed Central  Google Scholar 

  • Chan CC, Whitcup SM, Solomon D, Nussenblatt RB. Interleukin-10 in the vitreous of patients with primary intraocular lymphoma. Am J Ophthalmol. 1995;120:671–3.

    Article  CAS  PubMed  Google Scholar 

  • Chan CC, Rubenstein JL, Coupland SE, Davis JL, Harbour JW, Johnston PB, Cassoux N, Touitou V, Smith JR, Batchelor TT, Pulido JS. Primary vitreoretinal lymphoma: a report from an international primary central nervous system lymphoma collaborative group symposium. Oncologist. 2011;16(11):1589–99.

    Article  PubMed  PubMed Central  Google Scholar 

  • Farkas T, Harbour JW, Davila RM. Cytologic diagnosis of intraocular lymphoma in vitreous aspirates. Acta Cytol. 2004;48:487–91.

    Article  PubMed  Google Scholar 

  • Frenkel S, Hendler K, Siegal T, et al. Intravitreal methotrexate for treating vitreoretinal lymphoma: 10 years of experience. Br J Ophthalmol. 2008;92:383–8.

    Article  CAS  PubMed  Google Scholar 

  • Itty S, Pulido JS. Rituximab for intraocular lymphoma. Retina. 2009;29:129–32.

    Article  PubMed  Google Scholar 

  • Margolis R, Brasil OF, Lowder CY, et al. Vitrectomy for the diagnosis and management of uveitis of unknown cause. Ophthalmology. 2007;114:1893–7.

    Article  PubMed  Google Scholar 

  • Riemens A, Bromberg J, Touitou V, et al. Treatment strategies in primary vitreoretinal lymphoma: a 17-center European collaborative study. JAMA Ophthalmol. 2015;133:191–7.

    Article  PubMed  Google Scholar 

  • Shen DF, Zhuang Z, LeHoang P, et al. Utility of microdissection and polymerase chain reaction for the detection of immunoglobulin gene rearrangement and translocation in primary intraocular lymphoma. Ophthalmology. 1998;105:1664–9.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Ophthalmic Oncology/Retina Division, Wilmer Eye Institute, Johns Hopkins, 600 North Wolfe Street, Maumenee 744, Baltimore, MD, 21287, USA

    Mary E. Aronow

  2. Laboratory of Immunology, National Eye Institute, National Institutes of Health, 10 Center Drive #107, Bethesda, MD, 20892, USA

    Ian Yeung & Chi-Chao Chan

Authors
  1. Mary E. Aronow
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ian Yeung
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Chi-Chao Chan
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Chi-Chao Chan .

Editor information

Editors and Affiliations

  1. Advanced Eye Center, PGIMER, Chandigarh, India

    Vishali Gupta

  2. Ophthalmology, University of Nebraska Medical Center, Omaha, Nebraska, USA

    Quan Dong Nguyen

  3. Department of Ophthalmology, Pitié-Salpêtrière University, Paris, France

    Phuc LeHoang

  4. University of Lausanne, Lausanne, Switzerland

    Carl P Herbort Jr.

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer India (outside the USA)

About this entry

Cite this entry

Aronow, M.E., Yeung, I., Chan, CC. (2016). Vitreoretinal Lymphoma. In: Gupta, V., Nguyen, Q., LeHoang, P., Herbort Jr., C. (eds) The Uveitis Atlas. Springer, New Delhi. https://doi.org/10.1007/978-81-322-2506-5_128-1

Download citation

  • DOI: https://doi.org/10.1007/978-81-322-2506-5_128-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, New Delhi

  • Online ISBN: 978-81-322-2506-5

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation